Autoimmune Disorders and Hydroxychloroquine Guidelines

Autoimmune Disorders

• Autoimmune Disorders occur when the body's immune system attacks its own tissues.
• They are diagnosed through laboratory tests, particularly by identifying specific antibodies in the blood.
• The most common tests used to diagnose autoimmune disorders include Rheumatoid Factor (RF), Anti-citrullinated protein antibodies (ACPAs or anti-CCPs), Antinuclear antibodies (ANAs), Anti-double stranded DNA antibodies (anti-dsDNA), Anti-neutrophil cytoplasmic antibody (ANCA), Erythrocyte Sedimentation Rate/C-Reactive Protein (ESR/CRP), and Human Leukocyte Antigen B27 (HLA-B27).
• Treatment generally involves medications like hydroxychloroquine, steroids, or biologics to suppress the immune system.

Hydroxychloroquine Retinopathy Testing Guidelines

• Baseline eye exam within 1 year of starting hydroxychloroquine
• Annual screening starting after 5 years of use, unless maculopathy or major risk factors are present
• More frequent screening if maculopathy or major risk factors present
• Primary Testing: automated visual fields (10-2 White Light or 24-2 for Asian patients), OCT of the macula, Dilated fundus exam
• Adjunctive Testing: Multifocal ERG, Fundus autofluorescence
• Ineffective Testing: Fluorescein angiography (FA), Full-field ERG, EOG, Amsler grid, Color vision testing

Giant Cell Arteritis (GCA)

• GCA is a serious condition characterized by inflammation of the temporal artery.
• GCA commonly affects people over 50 years old, causing a sudden, severe headache, tenderness to palpation of the temporal artery, elevated Erythrocyte Sedimentation Rate (ESR), and abnormal temporal artery biopsy (TAB) features.
• Diagnosis requires the presence of at least four of the following criteria: age > 50, new headache, tenderness or decreased pulsation of temporal artery, ESR ≥50 mm/hr, abnormal TAB.
• Oral or IV steroids are the primary treatment, with a taper over months to years.

Arteritic Anterior Ischemic Optic Neuropathy (AAION)

• AAION is a sudden and severe loss of vision caused by inflammation of the artery supplying the optic nerve.
• Visual field defects primarily affect the periphery.
• Treatment consists of high doses of steroids to prevent vision loss in the other eye. Since the optic nerve is damaged, visual recovery in the affected eye is rarely complete.

Systemic Lupus Erythematosus (SLE)

• SLE is a chronic autoimmune disease that can affect various organs, including the skin, joints, kidneys, heart, lungs, and brain.
• SLE commonly causes fatigue, joint pain, skin rashes, and photosensitivity.
• Diagnosing SLE requires the presence of at least four out of eleven criteria, such as malar rash, discoid rash, photosensitivity, oral ulcers, arthritis, serositis, renal disorder, neurological disorder, hematological disorder, immunologic disorder, and antinuclear antibody (ANA).

Ocular Effects of Lupus

• Eye involvement in SLE, known as lupus retinopathy, can cause retinal vasculitis and retinal hemorrhages.

Rheumatoid Arthritis (RA)

• RA is a chronic autoimmune disorder that primarily affects the joints, leading to inflammation, pain, stiffness, and joint damage.
• The diagnosis requires the presence of four out of seven criteria, such as morning stiffness lasting at least one hour, arthritis involving three or more joints, arthritis of hand joints, symmetrical joint involvement, rheumatoid nodules, positive rheumatoid factor (RF), and radiographic changes characteristic of RA.

Ocular Effects of RA

• RA can cause scleritis, episcleritis, keratoconjunctivitis sicca, and corneal melting.

Sjogren's Syndrome (SS)

• SS is a chronic autoimmune disorder that primarily affects the exocrine glands, including the salivary and lacrimal glands.
• It causes dry mouth (xerostomia) and dry eyes (keratoconjunctivitis sicca).
• Symptoms include dry eyes, dry mouth, oral ulcers, fatigue, and joint pain.
• Diagnosis requires the presence of four points from the following: positive Anti-Ro/SSA antibodies (3 points), lip biopsy showing Focus > 1 foci/4mm^2 (3 points), ocular staining score ≥ 5 (1 point), Schirmer’s test (without anesthetic) ≤ 5 mm/5 min (1 point), and unstimulated salivary flow rate ≤ 0.1 mL/min (1 point).

SS Management

• Treatment often includes artificial tears, saliva substitutes, and medications that stimulate saliva production.

Spondyloarthropathies (SpA)

• SpA is a group of inflammatory diseases that primarily affect the spine and the sacroiliac joints.